TY - JOUR
T1 - Lymphomatoid Papulosis in Children
T2 - A Retrospective Cohort Study of 35 Cases
AU - Nijsten, Tamar
AU - Curiel-Lewandrowski, Clara
AU - Kadin, Marshall E.
PY - 2004/3
Y1 - 2004/3
N2 - Background: Lymphomatoid papulosis (LyP) is a rare entity, considered to be part of the spectrum of the CD30+ cutaneous lymphoproliferative disorders. About 10% to 20% of the adult LyP patients will develop an associated lymphoid malignancy. Only a few cases of LyP have been described in children, and the risk of associated lymphoid malignancies in these patients is not known. Objectives: To study the association between childhood onset of LyP and other malignancies and to determine the clinical characteristics in this subgroup of patients. Design: Retrospective cohort study. Setting: Referral center at a university hospital. Retrospective registry for patients with LyP of childhood onset (≤18 years). Patients: Thirty-five patients with childhood-onset LyP (19 boys and 16 girls) were interviewed by telephone using a standardized questionnaire. The median duration of follow-up was 9.0 years. All included patients were confirmed by histologic examination. Results: The age distribution was significantly different, with boys having an earlier onset of LyP (P=.03). Of the 35 LyP patients, 3 (9%) developed a malignant lymphoma; all were diagnosed as having non-Hodgkin lymphoma. Compared with the general population, patients with childhood-onset LyP have a significantly increased risk of developing non-Hodgkin lymphoma (relative risk, 226.2; 95% confidence interval, 73.4-697.0). More than two thirds of the patients reported being atopic, which is significantly more than the expected prevalence of atopy (relative risk, 3.1; 95% confidence interval, 2.2-4.3). Conclusions: Lymphomatoid papulosis presents similarly in children and adults, including the risk of lymphoid malignancies. Therefore, all LyP patients should be closely monitored throughout their lives.
AB - Background: Lymphomatoid papulosis (LyP) is a rare entity, considered to be part of the spectrum of the CD30+ cutaneous lymphoproliferative disorders. About 10% to 20% of the adult LyP patients will develop an associated lymphoid malignancy. Only a few cases of LyP have been described in children, and the risk of associated lymphoid malignancies in these patients is not known. Objectives: To study the association between childhood onset of LyP and other malignancies and to determine the clinical characteristics in this subgroup of patients. Design: Retrospective cohort study. Setting: Referral center at a university hospital. Retrospective registry for patients with LyP of childhood onset (≤18 years). Patients: Thirty-five patients with childhood-onset LyP (19 boys and 16 girls) were interviewed by telephone using a standardized questionnaire. The median duration of follow-up was 9.0 years. All included patients were confirmed by histologic examination. Results: The age distribution was significantly different, with boys having an earlier onset of LyP (P=.03). Of the 35 LyP patients, 3 (9%) developed a malignant lymphoma; all were diagnosed as having non-Hodgkin lymphoma. Compared with the general population, patients with childhood-onset LyP have a significantly increased risk of developing non-Hodgkin lymphoma (relative risk, 226.2; 95% confidence interval, 73.4-697.0). More than two thirds of the patients reported being atopic, which is significantly more than the expected prevalence of atopy (relative risk, 3.1; 95% confidence interval, 2.2-4.3). Conclusions: Lymphomatoid papulosis presents similarly in children and adults, including the risk of lymphoid malignancies. Therefore, all LyP patients should be closely monitored throughout their lives.
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U2 - 10.1001/archderm.140.3.306
DO - 10.1001/archderm.140.3.306
M3 - Article
C2 - 15023774
AN - SCOPUS:1542410115
SN - 0003-987X
VL - 140
SP - 306
EP - 312
JO - Archives of Dermatology
JF - Archives of Dermatology
IS - 3
ER -